Xanthogranulomatous osteomyelitis of atlanto-occipital region: an extremely rare clinical entity

• First reported case of xanthogranulomatous osteomyelitis in the atlanto-occipital region. • 54-year-old male presented with neck pain, stiffness, headaches, and fever. • Imaging showed a lytic lesion at the atlas and occipital condyle. • Successful surgical management using a transnasal endoscopic and microscopic approach. • Histopathological confirmation of xanthogranulomatous osteomyelitis emphasizes the importance of biopsy in rare spinal infections. Xanthogranulomatous Osteomyelitis is a rare form of chronic inflammation described in a handful of cases in the reported literature involving the long bones of the axial skeleton. To our knowledge, it has only been reported once in the spinal column. We report a case of a 54-year-old native Indian male presenting with two months of worsening neck pain, stiffness, headaches, and fever. Imaging revealed a lytic lesion involving the anterior arch and lateral mass of the atlas, extending to the right occipital condyle. Patient underwent transnasal supra and infra palatal approach with both endoscopic and microscopic techniques. A histological diagnosis of Xanthogranulomatous Osteomyelitis was made. We report what the authors believe to be the first case in the literature of xanthogranulomatous osteomyelitis affecting the atlanto-occipital region.